Glomus medium ear tumor
Last reviewed: 23.04.2024
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
The glomus tumor belongs to one of the varieties of paraganglia, which are an accumulation of hormonal-active and receptor cells having a common origin with the VNS. Distinguish chromaffin (that is, linked by salts of chromic acid) and nonchromaffin paraganglia. The former were united under the name "adrenal system"; they are functionally associated with the sympathetic department of the VNS, and the latter - with its parasympathetic department.
The largest paraganglia are the adrenal (adrenal medulla) and the lumbar aortic. There are also laryngeal, tympanic, jugular and other parangenia. Paraganglia include congestions of chromaffin cells in the form of glomus (nodules), including carotid, over-cardiac and other localization. Individual glomus tumors in macrostructure are clusters of arteriovenous anastomoses surrounded by a connective tissue capsule and divided into lobules and strands. In the cytoplasm of chromaffin cells, a large number of small granules containing adrenaline or norepinephrine are scattered. In non-chromaffin cells, the secretion of polypeptide hormones not related to catecholamines is suggested. In the glomus tumors, the vascular network is well developed; most secretory cells are adjacent to the walls of the vessels. The cells of the paraganglia terminate the centrifugal processes of the cells of the lateral horns of the gray matter of the spinal cord and the vegetative nuclei of the glossopharyngeal and vagus nerves. Nerve fibers penetrating into the paraganglia end with chemoreceptors that perceive changes in the chemical composition of tissue and blood. A particularly important role in chemoreception belongs to the carotid glomus located in the region of dividing the common carotid artery into the internal and external. Paraganglia are sometimes sources of development of tumors - paraganglia and chromaffin - or systemic diseases such as Barre-Masson's disease (syndrome), a manifestation of so-called active glomus tumors in the circulatory system that produce certain substances that cause common symptoms such as asthma attacks, tachycardia, arterial hypertension, fever, dry skin, dysmenorrhea, insomnia, a sense of fear and other phenomena of psychoemotional dysfunction, indicating the impact these substances in the limbic-reticular system of the brain. Many of these signs are characteristic of the glomus tumor of the middle ear.
What causes the glomerular tumor of the middle ear?
Normally, the jugular glomus is a kind of arteriovenous shunt located in the area of the jugular vein bulb at the jugular opening of the skull base (posterior laceration), consisting of vascular arteriovenous plexuses. As an independent nosological form, the glomus tumor of the jugular parangenlia took shape in 1945. Despite the fact that in structure this tumor belongs to benign tumors with slow development, it can pose a significant danger with respect to those disruptions that it can cause to adjacent vital organs at its distribution. This destructive effect is caused not only by the actual pressure of the tumor, but also by those still not thoroughly studied "caustic" substances that stand out on its surface and cause resorption with the tissues in contact with them. Being located primarily in the area of bulbi venae jugularis, the tumor in its development can spread in three directions, causing the emergence of three syndromes, corresponding to each of the directions presented in the figure.
Symptoms of the glomus middle ear tumor
The otiatric syndrome makes its debut by the appearance of pulsating blowing noise in one ear, varying in intensity or disappearing when clamping on the corresponding side of the common carotid artery. The rhythm of the noise is synchronized with the heart rate. Then there is a progressive one-sided hearing loss at first of a conductive nature, and when the tumor is invaded in the inner ear - and perceptually. In the latter case, vestibular crises that increase in intensity also appear, which usually result in the shutdown of both vestibular and auditory functions on the same side. Objectively, there are signs of presence in the tympanic cavity of blood or a vascular tumor that shines through the tympanic membrane in the form of a reddish-pink or cyanotic formation, often pushing the eardrum outwards. Further development of the tumor leads to destruction of the tympanic membrane and exit into the external auditory canal of tumor masses, red-cyanotic color, easily bleeding when probed with a probe probe.
With otoscopy, a cyanotic tumor of a fleshy consistency is found that occupies the entire bone part (and more) of the external auditory canal, dense and bleeding, pulsating and growing into the defect of the posterolar portion of the lateral wall of the overdrug, "bathing" in abundant purulent discharge. A tumor through aditus ad antrum can penetrate into the cells of the mastoid process or when spreading anteriorly - into the auditory tube and through it into the nasopharynx, imitating the primary tumor of this cavity.
The neurological syndrome is caused by the germination of paraganglioma in the posterior cranial fossa, where IX, X, XI cranial nerves are affected, causing the appearance of a ruptured hole syndrome through which these nerves leave the cavity of the skull, showing signs of their defeat: paresis or paralysis of the tongue on the corresponding side, articulation disorder , open nasal and liquid food in the nose (paralysis of the soft palate), swallowing, hoarseness, aphonia. In this syndrome, otiatric symptoms are absent or insignificant. In the case of further progression of the process, the tumor can penetrate into the region of the lateral cistern of the brain and cause the syndrome of MMU with lesions of the facial, pre-door, cochlear and trigeminal nerves. The spread of a tumor in the brain can trigger the onset of syndromes such as the syndromes of Berne and Sicar.
Berne's syndrome refers to alternating paralysis, develops as a result of damage to the pyramidal path in the medulla oblongata, and is manifested by contralateral spastic hemiparesis, homolateral paralysis of the palate and swallowing muscles with sensitivity disorders in the posterior third of the tongue, and paralysis of the sternum -directed and trapezius muscles): the turn and inclination of the head to the healthy side are difficult, the shoulder on the diseased side is omitted, the lower corner of the pathes deviate from the spine to the outside and up, it is difficult to shrug the shoulders.
Stahr's syndrome is manifested by the neuralgia of the glossopharyngeal nerve: a sudden intolerable daggerache on one side of the soft palate during the use of hard, especially hot or cold food, and also during chewing, yawning, and speaking with a loud voice; an attack of pain lasts about 2 minutes; pain radiates into the tongue, jaw, adjacent portion of the neck and ear.
Neurological syndrome may manifest as signs of increased intracranial pressure (congestive discs of the optic nerves, persistent headaches, nausea, vomiting).
Cervical syndrome by manifestation resembles the aneurysm of a large cervical vessel and is due to the presence of a pulsating tumor in the region of the lateral section of the neck.
The clinical course and symptoms of the glomus tumor of the middle ear are characterized by a long and slow development for many years, which in the untreated cases passes otiatric, neurological (defeat of the peripheral nerves), cervical, intracranial and terminal phases, germinating into the surrounding large veins and spaces of the MMU.
Diagnosis of the glomus middle ear tumor
Diagnosis of the glomus tumor of the middle ear is difficult only at the initial stages of the disease, but pulsating noise, one of the earliest signs of the glomus middle ear tumor, which occurs before penetration of the formation in its cavity, should always alert the doctor about the presence of this disease, and not just explain this noise by any defect in the carotid artery, for example by narrowing its lumen with an atherosclerotic process. The consistently occurring conductive hearing loss, signs of cochlear and vestibular dysfunctions, ruptured hole syndrome, cervical pseudoaneurysmal symptoms, and the above picture with otoscopy are of significant importance in diagnosis. Diagnostic methods are completed by radiography of the temporal bones in the projections of Schueller, Stenvers, Highway III and II, in which the disruption of the tympanum and the above-drum space, the widening of the lacerated hole and the lumen of the osseous part of the external auditory canal can be visualized on the radiographs.
Histological examination reveals closely adjacent to each other polygonal giant cells with different forms of nuclei and cavernous tissue.
Treatment of glomus middle ear tumor
Treatment of the glomerular tumor of the middle ear includes both surgical removal of the tumor and methods of physiotherapy (diathermocoagulation, laser evaporation of tumor tissue followed by radio or cobalt therapy). Operative intervention should be made as soon as possible and within the widest limits, by the type of petromastoid surgery.
What is the prognosis of the glomus middle ear tumor?
Depending on the time of diagnosis, the direction of tumor growth, its size and treatment, the glomerular tumor of the middle ear has from cautious to very serious prothorax. Relapses are very frequent.